Senin, 29 Maret 2010

CAMERON MOTT ... (an amazing little girl)

Living with Half a Brain

When Cameron Mott was three years old, she contracted a rare and deadly brain disorder; Rasmussen's Encephalitis. The disease is eating away at her brain and she's plagued by fits. Her parents are desperate and have opted for the only available cure, a radical high-risk operation to remove the diseased half of her brain.

Cameron lives with her sister Caroline and her parents Casey and Shelley in Jamestown, North Carolina.

For the first few minutes of every day, she's like any other six year old; lucid and able to play. But, within an hour of waking, her epilepsy strikes. Surges of electricity in Cameron's brain are causing her nervous system to misfire. Her seizures are tonic causing her muscles to stiffen and contract, and she temporarily loses consciousness.

With a prolonged seizure, her parents have to medicate her to bring her round. The seizures are caused by Rasmussen's Encephalitis, a debilitating condition that affects less than one in a thousand children. At her worst, Cameron has fifteen seizures a day. Her fits are so unpredictable, they rob her of any chance of a normal life.

Cameron has to wear a specialised helmet to protect her from the constant threat of head trauma. Doctors are unsure what causes Rasmussen's, but in each case the brain becomes chronically inflamed, destroying the brain cells. As the disease spreads through Cameron's right hemisphere it affects the opposite side of her body, gradually paralysing her left hand side and causing her mental faculties to deteriorate. The daughter that Casey and Shelley Mott loved was beginning to disappear.

Untreated, Cameron's disease will continue to eat away at her brain leading to severe handicap and brain damage. The only cure is a hemispherectomy, a radical operation to remove the diseased right-half of her brain. Dr George Jallo a neurosurgeon comments "For many years, this procedure wasn't practised because of the risks of the surgery".

Shelley and Casey have to weigh up the cost of removing one half of Cameron's brain. She'll be left with a hemiplegia, a stroke-like effect on the left side of her body. But, without the surgery the disease will continue to spread and she'll never be seizure free. The operation is the last resort.

Cameron Mott will be treated 400 miles away at a leading paediatric neuro facility; The John Hopkins Children's Centre in Baltimore. The hemispherectomy is one of the most radical procedures in neurosurgery. Dr George Jallo is a specialist in paediatric hemispherectomies; he performs up to nine each year. During the surgery, Cameron will be monitored by anaesthetist Marco Ramiro and the operation will take between seven and twelve hours.

As Dr Jallo removes the front of the skull, the damage beneath the dura mater becomes apparent. The membrane, normally translucent, is tough and opaque, a sign of inflammation. As he folds back the dura mater he gets a first glimpse of Cameron's brain "When I opened up the dura I could see the brain was atrophic, there were parts of it that were dying".

A hemispherectomy is a delicate and intricate operation. The surgeon must work piece by piece to navigate through the dense network of veins and arteries inside the brain. An accidental cut could cause a lethal haemorrhage. Jallo must send large chucks of brain to the pathology lab to confirm Rasmussen's

Jallo removes the right hemisphere, working systematically towards the back of the brain. The tissue that can't be removed in chunks is drawn out through a suction pipe until the right-side of Cameron's brain is emptied. In a crucial part of the operation the surgeon has to leave a small piece of the brain to protect the fragile area surrounding Cameron's spinal cord. But, leaving even a tiny part of the infected brain could lead the seizures to return. He must also sever the corpus callosum, the band of nerve fibres located deep in the brain that connected the two halves.

The cavity on the right side of Cameron's head will fill with cerebral fluid at a rate of one teaspoonful every five minutes. Cameron will be closely monitored. Excess build up of the fluid, a condition called hydrocephalus, can put dangerous levels of pressure on the remaining half of her brain. To prevent bleeding, a white powdered clotting agent is tipped into the space. After eight hours, the surgery is complete

For the next 48 hours, Cameron's head must be kept completely still so that the remaining half of her brain isn't dislodged. Cameron's journey to recovery will now begin.

Medical evidence shows that a young child's brain has the remarkable ability to reorganise itself, transferring skills normally undertaken by one half of the brain to the other. A process called plasticity. No-one knows exactly how this happens. The hope is that as the Rasmussen's has attacked the right hemisphere, it's functions will now shift to the left. All the doctors can do now is wait to see if the remaining left hemisphere will learn to move the left-hand side of her body and adapt fully enough to allow her to lead a full and active life.

Only 9 or 10 days later, Cameron is showing excellent signs of recovery. She is walking on a treadmill, where Dr Amy Bastion is trying to train her to walk without a limp "What we're interested in doing here is being able to train these kids from right after the surgery. These kids, initially of course, can't move their leg, but rapidly they begin to be able to stand and bear weight and walk, but their walking pattern is asymmetric, so we're trying to train out the limp, from the start, so she never establishes the abnormal pattern".

Cameron has shown extraordinary courage and its beginning to pay off. Each day brings new evidence that her brain is recovering.